Bilateral adrenalectomy, in which the adrenal glands are removed, has a bigger negative impact on the quality of life of patients with Cushing’s disease than other treatment options, a recent study suggests.
This may be due to the longer exposure to high levels of cortisol in these patients, which is known to greatly affect their quality of life, the authors hypothesize.
The study, “Bilateral adrenalectomy in Cushing’s disease: Altered long-term quality of life compared to other treatment options,” was published in the journal Annales d’Endocrinologie.
Cushing’s disease is caused by a tumor in the pituitary gland in the brain that secretes large amounts of adrenocorticotropic hormone, which, in turn, stimulates the adrenal glands to produce high levels of cortisol (a glucocorticoid hormone).
The gold standard for treating Cushing’s disease is the surgical removal of the pituitary gland tumor. However, 31% of these patients still require a second-line treatment — such as another surgery, radiotherapy, medical treatment, and/or bilateral adrenalectomy — due to persistent or recurrent disease.
Bilateral adrenalectomy is increasingly used to treat patients with Cushing’s disease, with high rates of success and low mortality rates. However, since the absence of adrenal glands leads to a sharp drop in cortisol, this treatment implies lifelong glucocorticoid replacement therapy and increases the risk of developing Nelson syndrome.
Nelson syndrome is characterized by the enlargement of the pituitary gland and the development of pituitary gland tumors, and is estimated to occur in 15-25% of Cushing’s patients who have a bilateral adrenalectomy.
Despite being cured with any of these treatment options, patients still seem to have a lower quality of life than healthy people. In addition, there is limited data on the impact of several of the treatment options on quality of life.
Researchers in France evaluated the long-term quality of life of Cushing’s disease patients who underwent bilateral adrenalectomy and compared it with other therapeutic options.
Quality of life was assessed through three questionnaires: one of general nature, the Short Form-36 Health Survey (SF-36); one on disease-specific symptoms, the Cushing QoL questionnaire; and the last focused on mental aspects, the Beck depression inventory (BDI).
Researchers analyzed the medical data, as well as the results of the questionnaires, of 34 patients with Cushing’s disease — 24 women and 10 men — at two French centers. The patients’ mean age was 49.3, and 17 had undergone bilateral adrenalectomy, while the remaining 17 had surgery, radiotherapy, or medical treatment.
Results showed that patients who underwent a bilateral adrenalectomy were exposed to high levels of cortisol significantly longer (6.1 years) than those on other treatment options (1.3 years). This corresponds with the fact that this surgery is conducted only in patients with severe disease that was not controlled with first-line and/or second-line treatment.
These patients also showed a lower quality of life — particularly in regards to the general health, bodily pain, vitality, and social functioning aspects of the SF-36 questionnaire, and the Cushing QoL questionnaire and BDI — compared with those who underwent other therapeutic options.
This and other studies support the hypothesis that these patients’ lower quality of life may be caused by longer exposure to high cortisol levels, and “its physical and psychological consequences, as well as the repeated treatment failures,” according to the researchers. Additionally, the presence of Nelson syndrome in these patients was associated with a significantly lower quality of life related to mental aspects.
The team also found that adrenal gland insufficiency was a major predictor of a lower quality of life in these patients, regardless of the therapeutic option, suggesting it may have a stronger negative impact than the type of treatment.
They noted, however, that additional and larger prospective studies are necessary to confirm these results.